Clonal Lymphoproliferations in a Patient With Common Variable Immunodeficiency

Common variable immunodeficiency (CVID) comprises a heterogeneous group of disorders of humoral immunity, characterized by marked deficiencies in serum immunoglobulins. Immune dysregulation causes susceptibility to recurrent bacterial infections, as well as autoimmune and lymphoproliferative disorders. Although the lymphoid cells comprising the atypical proliferations are often clonally related, their malignant potential and clinical significance differ from similar lesions in individuals with immunocompetence. Herein, we describe a Caucasian woman with CVID who over 7 years developed multiple clonal lymphoproliferative lesions, comprising a spectrum of morphologic characteristics. Many of the lesions harbored distinct clonal populations. Though a majority responded to conservative intervention, 1 lesion persisted, met the diagnostic criteria for diffuse large B-cell lymphoma, and responded well to conventional chemotherapeutic treatment. The patient subsequently developed additional lymphoproliferations, but the lesions were clonally distinct and responded to conservative therapy. The clinical course of this patient emphasizes the variable nature of lymphoproliferative lesions arising in patients with CVID and underscores an individualized approach to pathologic interpretation and diagnostic intervention.